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58 THE BRITISH JOURNAL O F SURGERY MULTIPLE POLYP1 OF THE COLON. BY SIR W. I. DE C. WHEELER, DUBLIN. POLYPOSIS OF the intestinal tract is not b y any means a.s rare as was formerly supposed. The relative frequency of polypi in the rectum is probably more imaginary than real, for the ease of diagnosis in this situation is in sharp contrast to the difficulties encountered when other portions of the alimentary canal are invaded. Difficulties, both in diagnosis and treatment, were encountered in two recent cases of polyposis of the colon which have stimulated the desire to place them on record and t o review some of the literature. I n the first case the condition was not recognized either before or during the course of two operations, and yet the findings were so definite that, if a second similar case mere encountered, recognition during operation in all probability would present little or no difficulty. CASE REPORTS. Case 1.-Polyposis with Symptoms of Ulcerative Colitis.-In May, 1924, a girl, age 26, was admitted t o Mercer's Hospital, with a history of constipation, followed after the use of laxatives by loose bloodstained motions which had persisted for two years. Stated briefly the symptoms were those of ulcerative colitis. Palpation revealed tenderness over the right segment of the colon, and a n elongated tumour could readily be felt. The temperature ranged from 99" t o 102", running normal for two or three days a t a time. Loss of weight, ansemia, pyrcxia, and the presence of a tumour led t o the clinical diagnosis of a malignant or tuberculous tumour of the cxcum. The possibility of actinomycosis, colospasm, or a specific lesion was considered. No definite information was forthcoming from X-ray examination ; the report stated t h a t twcnty-four hours after the barium meal the bowels had moved several times, and very little barium remained in the colon. Some was in the transverse and some was in the pelvic colon, but the relationship of the tuniour t o the bowel was not defined ". FIRSTOPEEATION.-After some preliminary treatment with arsenic a i d by blood transfusions the abdomen was opened. The colon presented a quite unfamiliar appearance. From just above the ciecum (which was normal) t o the junction of the descending with the pelvic colon7 the walls wcre hyperaemic, and infiltrated t o such an extent as to convey the impression t h a t any rough handling or angulation would break it in two. There wcrc no adhesions. The ileum and pelvic colon were shortcircuited, an appendicostoniy was established, and, after removal of a small ])ortion of the bowel wall for examination, the abdomen was closed. The late Professor O'Sullivan reported that the specimen showed no signs of malignancy, but septic inflammation of the mucous meinbralie was present. LL MULTIPLE POLYP1 OF THE COLON FIG.39.--Polyposis of tho colon. Drnwing inado Iinmedintely nfter reniovnl. Thc c m m i and luwer portion of the pelvic colon wore free from polypi. Tlw spcciineli weigliecl 2 i Ib. 59 60 THE BRITISH JOURNAL O F SURGERY After three weeks' irrigation through the appendicostomy the patient was discharged from hospital to the country, and told t o report again in six months. On re-admission a t the end of this period the tumour 011 the right side coitld still be fclt. The S - r a y report was as follows :-After six hours the czcum was incompletely filled and found very irregular in outline. The bowels had moved several times, and only traces of barium werc present in the ascending, transverse, and descending colon. A large, oval-shaped, barium-filled sac was shown lying obliquely in the pelvis, and more to the right than the left side. As this barium-filled sac was not present a t the first X-ray examination, i t was assumed that the barium collected a t the line of the previous ileocolic anastomosis. For five months pain and diarrhea had continued, but the hiemorrhage which had been present before operation had now ceased. SECOND OPERATION.-T~~ abdomen was again opened. Neither the appearance nor the feel of the colon had changed ; there was the same rigidity of the walls from a level above the ciecuni to the pelvic colon ; these two postions were normal, and in striking contrast t o the remainder of the bowel. A long tube was passed up the rectum and guided through the anastomosis betwe&i the ileum and pelvic colon. The colon was removed from the ileoczecal junctioii t o the line of anastomosis. The specimen weighed 24 lb. (The weight of the average normal colon is approximately 1 lb.) When opcned, myriads of polypi were found studded over the mucous membrane without interval from a line just above the caxiim t o the lower portion of the pelvic colon (Fig. 39). On the third day after operation the patient became distended, the tube passed through the ileocolic anastomosis did not drain, and ominous black vomiting supervened. Lavage of the stomach and efforts to establish drainage were unsuccessful, and the patient died. An autopsy was refused. The polypi on section were of the adenomatous type. Case 2.4nfantilism due to Polyposis of the Colon.-The second case was seen in March, 1925. The patient was a girl, 16 years of age, stunted 40.--1nfanti1ismin and dwarf-like, but not emaciated. Since early a girl, age 16, with polyposis childhood she had suffered from diarrhaa and the of the colon. passage of blood in the stools. Her height on admission to hospital was 4 ft. 5 in. and her weight 4 stone 13 lb. (The average normal height a t this age is 5 f t . 1 in., weight '7 stone 8% lb.) The breasts were undeveloped, and there was an absence of asillary and pubic hair (Fig. 40). Mentally she was alert and normal. MULTIPLE POLYP1 OF THE COLON 61 Tn the course OF roiitiiie esamiiintion tlie differential blood-count was iiotcwortliy owing to tlie great iiic~easeof the eosinophils, which raised ill the iiiiiid of tlic pathologist a suspicion of the presence of intcstiaal parasites. 'l'lie wliite blood-cclls were as follo~w: polyniorplis 22, Iylnpliocytes 59, large i ~ i o ~ i o ~ ~ ~ icells c l c a5r, eosinophils 14 ; poikilocytosis marked ; 110 iiiicleatecI rcd cells. Tiifantilisin has bceii clefiiial as " ail aiionialy of dedopnieiit cliaracterizecl by the pcrsisteiice of the iiioqhologhrl clmactcrs of childhood in an iuclividual wlio hits rcachetl or passed the age of piibeity " (Zuiidel). It is iiot surprising to liiid iiifnntilisni nssocititcd with polyposis of tlic c*oloii.but so f w as cnii be ascertained t.liis is tlic OIII>~ case 011 record. Apart from the so-called idiopathic in fantilisni, a i i t l the infantilism diie to general clisvirscs siirli as syphilis s i r c l tuberonlosis, a i d t l i ~ form t associated with the iiit.eriiii1secretion ol' itl)iiorinnl glaiicls, cliroiiic tlitirrlima fro111 vtirioiis causes appears to be responsible for tlie coiitlitioii in the majority of cascs. Ryroni Brainwell1 calls atteiitiou t o infantilisiii of paiicreatic origin in which diarrhwa is tlic proiiiiiieiit syiiiptom, and i t is interesting t.o nett that such cases :ire cured by the ;uliiiiiiist ration of pancreatic estract. hIoorIieac1~reviews t.lic literature and disciisses rases of infttiitilism -panci~atic and intestii~al. After a very complete description of a post-niorteni on a girl of IS, tliis latter writer states : " To siiiii 111). the ap1)areiitlp priin;iry ~~ttliological cliiilige in this rase w t s ctrtitrr~l of tile co~ollalld l)robabiy or tile siiiall intestine, aiid iii consequelice one inay regard t.lie case 8 s one of iuidoubtecl iiitcstiiial iiifaiitilisiii In the case under rcview the polypi in\-:~Icdthe rrctiini and c o d d easily be prolnpsed, I)iit- it wiis only dnriiig the course of two operations t.liat tliey wcrc found to estciid of 4 ~ ~ up~vards tliroiigli tlie desceiidiiig coloii and s~uddedover wit11 polypi. traiisvcrse colon, and probably into the aseelidiiig aiid crucal seginciit. Proctoscopic esariiiiiatioii in this, a s in the first case iiieiit.ioned, was unsatisfactory ; it was impossible, after passing through a 1 1 area of polypoid iiiiicoiis nictiihriiiie, to obt,aiii 11 rleilr visual iield owing to tiIe llrcsellce or Illis, lllllclls, alld bioori. Finsr Oimfa.riox.-~Iarch 3, 1035. The sphincter tvns diltited. The iiiiicous menibrane of the rectum was casily estruded, and was foulid covered wit11 sinall polypi. Tlic iiiiicoiis iiieiiibraiie was raised froin the subiiiucous coat by injections of iiorocain and adrenalin, and a wide cuff \vas removed, ". 7; ; 62 THE BRITISH JOURNAL O F SURGERY isolated polypi were cauterized, aiid the dividccl niticous membrane was sutured end t o end. The interior of the bowel could be reached to a high level ; but a t the conclusion of the operation polypi could be felt still higher u-ith the tip of the finger. SECONDO P E R - ~ T I O X . - ~ l l l ~ 30, ~ 1925. The pelvic colon was exposed through a left parmiediaii invision. A loop was opened for exploration, and the mucous nieiiibraiie mas found covered with polypi. The loop mas niobilized, and the limbs were sutured in parallel fashiou and fixed to the abdominal wall by tlie mcthotl of Rlikulicz. The estruded portion, measuring S $ in., was removed after n few days with the cautery. Numerous polypi FIG.42.-Polypi cstriidcd through the culostotny after partial colectoiny. were found on opening the portion renioved, but thcy were not diffuse, and the infiltration and loss of flexibility found in the first cnsc were in consequence absent (Fig. 41). A fiiigcr passed into the lower liiiib could detect 110 trace of further growths ; but 011 esaniiriillg the upper limb pedunculatecl polypi could be felt as high as the splenic flexure. I n a few days numerous polypi appeared through the upper limb of the colostomy wound, and were removed with the cautery. A week later, further polypi were cstrucled and removed in the same way (Fig. 42). After an interval of some weeks capillaries containing radium emanation were inserted in the end of a rubber tube and passed through the colostomy t o as high a level as possible. The tube was removed little by little so that the emanation would be brought into contact with a wide area of mucous coat. Finally, all MULTIPLE POLY PI O F THE COLON 63 trace of blood disappeared, and the colostomy was closed by crushing the spur and the introduction of a few sutures. In January, 1926, the mother reported that the child was progressing well ; she had put on nearly a stone weight and had grown one inch in ten months. Bleeding and diarrhea had ceased. In March, one pear after the commencement of treatment, the bloodcount approached normal. Eosinophils had fallen to 1 per cent, polymorphs had risen t o 75 per cent, large mononuclears 15 per cent, lymphocytes 9 per cent. Pubic and axillary hair had appeared, and there were further signs of general development. PATHOLOGICAL REPORT.--A~~ polypi appear in section as simple adenopapillomata. No sign of malignancy. DISCUSSION. Proctoscopic and X-ray examinations in the two cases under review were of little avail in making a diagnosis. In the first case the rectum and lower portion of the pelvic colon were unaffected, and above this level the field was obscured by fluid faxes, mucus, and blood. X rays confirmed the clinical picture of ulcerative colitis, and later showed the ileocolostomy, but did not go further. In the second case multiple polypi were present in the rectum. Kiimbers of them attached to a prolapsed mucosa could be protruded through the anus. The field of vision in this case was also obscured when the proctoscope was further introduced along the polypi-bearing mucosa. Barium given through the colostomy opening was not retained sufficiently to give a satisfactory X-ray picture. Reliance is, however, placed on these two methods of examination by Struthers and other authorities. Struthers3 opens a most illuminating paper by alluding to Rontgen-ray examinations and the diagnosis of multiple polypi before operation or death. All authorities are agreed as to the association between ulcerative colitis and polyposis, and in this connection the possibility of chronic ulcerative colitis in children should be borne in mind. In the second case the symptoms of colitis appear to have commenced a t about the age of 10. helm hot^,^ in recording five cases, the average being under 10 years, says he has failed to find a single article on the subject of this condition of ulcerative colitis in childhood. He thinks it was the terminal stage of ulcerative colitis of adults which was described by Rokitansky, Virchow, and others as polypi of the colon. According to some authorities the origin is congenital, and others lay stress on the familial type. Pennant,5 commenting on the first case I have mentioned in this paper, describes the post-mortem on a case of polyposis of the colon. The patient's brother was admitted six months later to hospital with a malignant growth in the left iliac fossa. A colostomy was performed, and the colon was found loaded with polypi similar to those found in the colon of his brother. A third brother came to visit him in hospital, and complained of the frequent passage of blood in the stools. The mother died of cancer a t an early age, and a sister of carcinoma of the uterus a t the age of 24. There are many references in the literature to intestinal polyposis. Hewitt and Howard,6 of Cleveland, refer to cases published as far back 64 THE BRITISH JOURNAL O F SURGERY as 1721, 1532, 1839, and 1861, and two more kecent cases recorded in 1905 and 1913. The formation of polypi following chronic ulcerative colitis is emphasized. The following cases are mentioned as illustrative of many interesting points :A woman, age 36, under the personal observation of Hewitt and Howard, in about the year 1905, had been afflicted for a considerable time with severe diarrhoea, and died in hospital a few weeks after admission. A t the post-mortem examination the only lesion of importance found was in the colon. Here the entire mucous membrane, from the ileocaxal valve to the rectum, was uniformly deeply ulcerated, with ragged tags of mucosa scattered widely. -4 second case, a man, age 40, was admitted to the Cleveland City Hospital in 1913. Diarrhoea was again the outstanding feature ; the temperature was variable, hut never above lolo. The patient died in hospital. At post-mortem examination, the mucosa of the lower two-thirds of the ileum was injected, edematous, and its surface covered with a thin, greyish layer of exudate. In the lower portion of the ileum, near t h e ileocecal valve, and in the cecum, there were single ulcers, which are minutely described. On the base of several of the largest ulcers there were attached islands and tags of miicosa and submucosa. The wall of the mucosa was thickened, rather fibrous, and stifflyflexible. In the ascending and transverse colons there were many small tufts of mucosa attached by slender pedicles easily detached by passing the finger over the surface. Just above the sigmoid flexure there were a few small polypoid projections ; they were stubby and attached by a relatively thick pedicle ; and, above this again, there was a cluster of large and long polypoid masses. The sigmoid flexure itself was the site of many projections, becoming more numerous as they extended downwards t o the external sphincter, but in all there were not more than forty such projections. Hen-itt and Howard thought, from a study of these two cases, that the islands and tags of mucosa and submucosa had been the source of the polypi, which appeared t o depend for their preservation 011 the blood-vascular arrangement, for i t was noted that the polyps in the rectum were situated along the side of the intestinal wall, while higher up and in the colon the polyps were situated along the line of attachment of the mesentery, an arrangement that coincides with the blood-supply of the parts. It is assumed that the beginning of the disease is a general ulcerative colitis. The ulcerative process is of such a character that portions of the mucosa and submucosa adjacent t o the primary arterial branches are preserved, and these portions remain as ragged tags scattered over the surface af the colon. As the ulcers heal, these tags become smoothed off, and remain as rounded sessile elevations, or as polypoid projections of the mucous surface. Later on, as cicatrization proceeds, the orifices of certain of the tubules situated in and between the polyps may become occluded, and retention cysts form, giving rise t,o what Virchow called ‘colitis polyposa cystica’. It is the end-stage of colitis polyposa. By some authorities a subtle distinction is made between adenomata, papillomata, and true polypi ; but i t appears likely that one is but a stage in the development of the other. In the Proceedings of the Boyd Society of Medicine, 1914, cases are recorded by Ivor Back, Gordon Watson, Norbury, and Furnivall. Back’s patient was a girl, age 24. The condition was diagnosed, microscopically and clinically, as carcinoma of the rectum. A colotomy was performed, and adenomata were found extending u p into the transverse colon, and they subsequently bulged through a colotomy opening. About a MULTIPLE POLYP1 OF THE COLON 65 year later she put on weight rapidly, and the polypoid excrescences had disappeared from the rectum and from the region of the colotomy wound. In Gordon Watson's case there were numerous edematous polypi throughout the large intestine, with carcinomatous change in the sigmoid flexure. After resection of the sigmoid the patient ultimately died of hemorrhage, and secondary growths were found in the lumbar glands and the liver. Norbury's case was one of multiple polypi of the rectum and colon, with prolapse of the bowel. The rectum and pelvic colon were studded with small sessile polypi which were afterwards found to extend as high as the descending colon. Fixation of the bowel to correct the prolapse, with appendicostomy, was the operation performed. In a discussion which followed, Lockhart-Mummery said that he believed that the only satisfactory treatment for these cases was complete excision of the entire colon after an ileorectostomy. The late J. B. Murphy7 recorded a case of polyposis of the sigmoid, and says that the etiology of intestinal polypi, like that of the common wart, is shrouded in mystery. Whence they come, how they go, is like the riddle of the Sphinx. Quoting Carroll, of the Mayo Clinic, he says that intestinal polyposis is a comparatively rare disease. Polypoid growths may occur at any point along the gastro-intestinal tract; but in the majority of cases they are found in the large intestine, usually a t its turning points, and in the rectum. A diagnosis can be made only when the polypi are seen or felt. In Murphy's case the microscope showed adenoma with suspicious areas of active cellular proliferations. Lockhart-Mummerys describes a case of complete resection of the large bowel for multiple adenomata. Numerous adenomata were found in the rectum and sigmoid which extended as high up as the czcum and all through the transverse colon. The specimen showed multiple adenomata of a simple character throughout the entire large intestine. Struthersg illustrates two specimens which appear almost identical with the specimen illustrated in Fig. 39, but in boih his cases an adenocarcinoma was found in the rectum. He gives an excellent reviewlo on what he calls " multiple polyposis of the gastro-intestinal tract ". Eighty-four cases in all are considered, two of which showed multiple polypi in the small intestine, an extremely rare condition. The various portions of the gastro-intestinal tract involved, according to this writer, are as follows : stomach, 4 cases ; czcurn to the rectum, 3 ; rectum and sigmoid, 2 ; hepatic flexure and small intestines, 1 ; small intestines, 1 ; transverse colon, splenic flexure, and descending colon, 1 ; descending colon and sigmoid, 1. Copell publishes one case of multiple papillomata of the small intestine causing recurrent intussusception in an adult ; and Mills12 reports a case of multiple polypi of the stomach, a condition which is generally regarded as of great rarity. The question of ultimate malignancy is discussed, and Lockhart-Mummery is quoted as having said that almost all recorded cases of multiple polypi of the colon eventually became malignant, and that this was the factor to be reckoned with in treating these cases. Erdmann and Morris13 state that the disease has a marked prcdilection VOL. XIV.-NO. 53. 5 66 THE BRITISH JOURNAL O F SURGERY for the male sex, but this statement is not borne out by a reference to the published cases. Attention is called by these writers to tlie fact that the adenomatous type of polypi is most frequent in the large intestine, usually in a multiple and widely disseminated form. The malignancy incidence they state is 43 per cent (Soper). Soper14 calls attention to the fact that L L comparatively few cases of multiple polyposis of the colon are reported in literature He analyses the grand total of 61 cases, and calls attention to the tendency for the growths to occur in the same family. A case is described (the second on record) in which the entire colon was successfully resected for polyposis. In view of the distribution of the polypi in C d e 1, it is interesting to note that, according to Soper, the rectum and sigmoid are involved in 95 per cent of all cases of polyposis of the colon. He thinks that sigmoidoscopic examination is the only means by which an accurate diagnosis can be made. T. S. Swan15 resected a portion of the transverse colon by the Mikulicz method for the removal of a papillary adenoma. The diagnosis was made by exploration. Diarrhea with the passage of blood was the outstanding symptom. ". CONCLUSIONS. 1. There is a close association between ulcerative colitis and polyposis. Ulcerative colitis occurs in children as well as in adults, 8. The majority of cases sooner or later become malignant. 3. A condition of infantilism may result from polyposis of the colon in early life. 4. Polyposis of the colon cannot be diagnosed unless the polypi are seen or felt. Satisfactory X-ray and proctoscopic examinations are only possible in a proportion of cases. 5. When multiple polypi of a very diffuse nature are present in the colon there is a characteristic infiltration and want of flexibility in the walls which is unlike any other pathological condition. When handling the colon the increase in weight is very striking. 6. The prognosis is bad unless colectomy is performed. Ileostomy, czcostomy, or appendicostomy, followed by irrigation, has been successful in a few cases. REFERENCES. Edin. Med. Jour., 1015, May. BRAMWELL, BYROM, z M ~ T. G., ~ Dublin ~ Jour. ~ Med. ~ Sci., ~ 1920, ~ Jan. , ~ S T R U T ~ EMayo R S , Cfinics, 1923. HELMHOLZ, Ibid. PENNANT, D. H., Bn'f, Med. Jour., 1025, i, 856. HEWITTand HOWARD, Arch. of IntmtaZ Med., 1915. ' MURPHY, J. B., Clinics, 1916, June. 8 L o ~ ~ ~ ~ J. ~P., ~PTOC. - Roy. M uSOP. ~ Med., ~ ~1819. ~ ~ , a STRUTHERS, Ann. 01Surg., 1920, Dec. STRUTHERS, surg. Gynecol. and Obst., 1924, May. l1 COPE, V. ZACHARY,Brit. Jour. Surg., 1922, ix, 558. l 2 MILLS, G . PERCIVAL, Brit. Jour. Surg., 1922, x, 226. l3 ERDMANN and MORRIS, Surg. Gynecol. and Obsl., 1925,.April, 460. SOPEU,Amer. Jour. Med. Sci., 1916, March, 405. SWAIN, T. S., Radiology, 1925,Jan., 55.